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Abstract
One hundred and twelve family histories, with mitochondrial cytopathies in which (‘vertical’) transmission from at least one generation to the other occurred, were reviewed. A paternal versus maternal transmission ratio of 2:3.4 was found. The mitochondrial cytopathies with ‘vertical’ transmission were split into a group with chronic progressive external ophthalmoplegia (CPEO) and a group without CPEO (83 and 29 families respectively). The paternal versus maternal transmission ratios amounted to 1:1.2 for the CPEO related cytopathies and 1:3.3 for the non-CPEO-related cytopathies with the exclusion of X-chromosomal myopathy and myoclonus epilepsy with ragged-red fibres (MERRF). For MERRF a mitochondrial heredity could not be excluded. For the other hereditary mitochondrial cytopathies a Mendelian form of hereditary transmission is most likely. For the CPEO related transmittable cytopathies an autosomal dominant heredity of a pleiotropic gene is the most probable form of transmission.